SARCOIDOSIS

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     1  SARCOIDOSIS Rheumatology Module Anna Mae Smith, MPAS, PA-C
     2  Definition Multisystem granulomatous disorder What’s granulomatous??? Mass or nodule composed of chronically inflamed tissue. Non-infectious multisystem disease of unknown cause, commonly affecting young and middle-age adults.
     3  Presentation Bilateral symmetrical hilar lymphadenopathy Pulmonary infiltrates Skin lesions Eye lesions(uveitis) Other organs may be involved, including liver, spleen, lymph nodes, heart, and central nervous system.
     4  Immunopathology Sarcoid granulomas consist of focal accumulations of epitheloid cells, macrophages, lymphocytes, mainly T cells! T lymphocytes…slightly B cells in the circulatory system.
     5  Epidemiology Increased prevalence found in Scandinavians, Japanese, Irish females, and African American women 30-80 per 100,000 Predominant age: 20-60 years Women > men Course worse in African Americans than whites
     6  Differential Diagnosis Infectious granulomatous disease such as tuberculosis and fungal infections Foreign body reactions Lymphoma Other malignancies associated with lymphadenopathy Berylliosis
     7  Presentation Present with pulmonary symptoms or found on x-ray! Fatigue Weight loss
     8  Presentation Cough Shortness of breath Skin (new lesions) Pain or irritation of eyes General fatigue, malaise Fever Night sweats Bell's palsy
     9  
     10  Bilateral hilar lymph adenopathy Characteristic feature of sarcoid Often symptomless but may have associated dull, ache in chest, malaise & fever
     11  Pulmonary Infiltration More progressive & leads to dyspnea…cor pulmonale…death Honeycomb appearance on chest x-ray! PFT’s restrictive pattern
     12  
     13  Skin Erythema nodosum Bilateral symmetrical hilar lymphadenopathy + erythema nodosum = sarcoidosis!!!
     14  
     15  Bone & Joint Arthralgias Bone cysts (especially in the digits) Swelling
     16  
     17  Acute Sarcoid Arthritis Migratory, Additive, Symmetric Polyarthritis (usu. Ankles & knees) Monoarthritis very rare Minimal effusion Minimal Inflammation Synovial Bx- Mild inflammation
     18  Cardiac Ventricular dysrhythmias Conduction defects Cardiomyopathy with CHF Hypercalcemia
     19  labs X-ray CBC - normochromic, normocytic anemia, Lymphopenia, leukopenia Chem20 - calcium & hypergammaglobulinemia Bronchial Biopsy ACE PFT
     20  CXR staging Routine chest roentgenograms are staged using Scadding's classification: Stage 0 = normal Stage 1 = hilar adenopathy alone Stage 2 = hilar adenopathy plus parenchymal infiltrates Stage 3 = parenchymal infiltrates alone Stage 4 = pulmonary fibrosis
     21  Special Tests Abnormal liver function, especially increased alkaline phosphatase is frequently encountered Hypercalciuria occurs in up to 10% of patients, with hypercalcemia less frequent Kveim-Siltzbach skin test can be performed. This test is fairly sensitive (approximately 80%) and highly specific (> 95%)
     22  Be careful... Prednisone will lower serum-angiotensin converting enzyme Hyperthyroidism and diabetes will increase serum angiotensin converting enzyme level
     23  Treatment Hilar lymphadenopathy alone with no evidence of lung parenchymal involvement…NO treatment! Steroids!! 30mg qd x 6 wks than 15mg qd x 6-12 months Topical too!
     24  Alternative Drugs Methotrexate 10 mg/wk hydroxychloroquine (Plaquenil) 100-200 mg per day azathioprine 50-100 mg per day chlorambucil 0.1-0.2 mg/kg.
     25  Prognosis If bilateral hilar lymphadenopathy…80% remission rate 80% of patients will have spontaneous resolution within two years 10% will have significant fibrosis but no further worsening of disease after 2 yrs 10% (higher in some populations, including African Americans) will have chronic dz.